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Description : Neuroglial tumors

Tags : Brain Tumors,Brain,Tumors

Published on : Oct 22, 2020 | Views : 3401 | Downloads : 9

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Neuronal and Glioneuronal Tumors Dr.N.Suriyaprakash JR , Dept of RD , GKMCH.
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CLASSIFICATION Neuroepithelial tumor encompasses all neoplasms that are derived from glial or neuronal precursor stem cells Pure Glial Tumors Glioneuronal Tumors Neuronal Tumors
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GLIONEURONAL TUMORS Less common than pure glial neoplasms WHO grade I Associated with seizures Less biologically aggressive than other glial tumors More favorable prognosis Anaplastic gangliogliomas are the only tumor that are assigned WHO grade III.
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Ganglion cell tumor - Ganglioglioma Desmoplastic infantile tumors Dysembryoplastic neuroepithelial tumor (DNET) Rosette-forming glioneuronal tumor Papillary glioneuronal tumor Diffuse leptomeningeal glioneuronal tumor
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GANGLION CELL TUMORS Benign, well-differentiated neoplasms characterized by the presence of dysplastic ganglion cells Gangliogliomas - Well-differentiated, slow-growing tumor composed of dysplastic ganglion cells and neoplastic glial cells Gangliocytomas - Ganglion cell tumors that demonstrate exclusive ganglion cell composition
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GANGLIOGLIOMA Well-differentiated, slow-growing tumor Biphasic tumor with variable combination of neuronal and glial elements Etiology and Genetics ○ Both components share common progenitor stem cell ○ BRAF V600E mutation ○ IDH1(-), 1p/19q not codeleted Turcot syndrome Neurofibromatosis type 1 & Neurofibromatosis type 2.
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Pathology ○ Dysplastic ganglion cells + Neoplastic glial cells ○ Superficial cortico-centric ○ Solid or mixed cystic/solid ○ Temporal > frontal lobes > parietal > brainstem > ventricles ○ Usually noninfiltrative
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Clinical Issues ○ Most common mixed glial-neuronal neoplasm ○ 1% - primary brain tumors but 10% in children ○ Common presentation = Seizures ○ Children and Young adults
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Imaging Features Cortically based superficial parenchymal lesions that have two general imaging patterns: Well-defined solid or partially cystic mass with mural nodule Diffusely infiltrating mass with ill-defined borders and patchy enhancement
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CT findings Varying attenuation on NECT Cystic component - 60% of cases Well-circumscribed hypodense cyst with isodense mural nodule - 30% Calcification - 30- 50% Hemorrhage is rare 50% of GGs enhance following contrast administration. Patterns vary from solid, rim, or nodular to cystic with an enhancing nodule
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MR Findings GGs are hypo- to isointense on T1WI and hyperintense on T2/FLAIR. Surrounding edema is generally absent Focal cortical dysplasia adjacent to the tumor. Enhancement - Heterogeneous. The classic pattern is a cystic mass with an enhancing mural nodule. Homogeneous solid enhancement also occurs. Ill-defined, patchy enhancement is atypical and associated with a worse clinical outcome
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Case 1 : 15 years / MC with epilepsy
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Case 2 : 30years / Female with seizures
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Differential Diagnosis Diffuse astrocytoma – No enhancement Pilocytic astrocytoma Pleomorphic xanthoastrocytoma Dysembryoplastic neuroepithelial tumor Oligodendroglioma
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PILOCYTIC ASTROCYTOMA Cerebellum Presentation – Headache , nausea …. Multiple cranial nerve palsies Calcification +/- Cystic PA hyperintense on both T1 and T2. No FLAIR suppression. Variable cyst wall enhancement GANGLIOGLIOMA Temporal lobe Presentation – Seizures Calcification – Common Cystic part is hypointense on T1 and hyperintense on T2 No cyst wall enhancement
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PLEIOMORPHIC XANTHO ASTROCYTOMA Suprasellar region – Hypothalamus , Optic Chiasma Presentation – Increases ICT , Failure to thrive , Diencephalic syndrome Calcification – Rare Intratumoral haemorrhage is common CSF dissemination is common – Worse prognosis GANGLIOGLIOMA Temporal lobe Presentation – Seizures Calcification – Common Haemorrhage is rare Rarely metastazize
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ANAPLASTIC GANGLIOGLIOMA Most are malignant transformation of previously diagnosed benign GG WHO grade III Pathology ○ Dysplastic ganglion cells + Anaplastic glial component ○ Increased cellularity, mitoses ○ Vascular proliferation, necrosis Imaging Findings ○ Atypical location common (i.e., deep rather than cortical) ○ Often larger, more infiltrative/poorly demarcated
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DESMOPLASTIC INFANTILE TUMOR Benign cystic lesions of young children that often have a radiologically aggressive appearance. Two forms of desmoplastic infantile tumors ○ Desmoplastic Infantile Astrocytoma (DIA) ○ Desmoplastic Infantile Ganglioglioma (DIG) WHO grade I
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Large bulky supratentorial masses Frontal and parietal lobe – MC site Mixed cystic – solid tumors involving adjacent superficial cortex and leptomeninges
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Pathology • Rare benign glioneuronal tumor Low-grade spindle cells in prominent desmoplastic leptomeningeal stroma Neuroepithelial cells (can appear relatively poorly differentiated) ○ Neoplastic astrocytes (DIA) ○ Variably mature neuronal component (DIG) • Ki-67 index usually < 2%
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Clinical Issues Presenting within the first year of life. Increasing head circumference with tense bulging fontanelles in a lethargic infant with "sunset eyes" is the typical presentation. DIA/DIG has a benign prognosis and very rarely metastasizes. Gross total resection generally results in long-term survival.
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Imaging features Massive, heterogeneous, peripherally located mixed cystic-solid supratentorial mass. Cystic portion is usually located relatively deep inside the hemispheric white matter Solid portion is typically peripheral directly abutting the dura. CT shows a cystic hypoattenuating portion with a mixed density solid component. Calcification +/-
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MR - Large, often multilobulated or septated cystic mass with a solid plaque-like dura-based component. The solid mass is often iso-/hypointense on T2WI and enhances strongly but often heterogeneously. Adjacent dural thickening - dural tail sign is common. DIA/DIGs may exhibit restricted diffusion
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Differential Diagnosis • Teratoma PNET • RELA-fusion ependymoma – Older children , Haemorrhage , Calcification Rarely large Solid component does not usually abut the dura
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DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR (DNET) Benign cortically based lesion characterized by a multinodular architecture WHO grade I neoplasms Etiology • FGFR1, BRAF mutations Location ○ Supratentorial, superficial ○ Intracortical ○ Temporal lobe most common site
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Size and Number -DNETs are generally solitary lesions and vary in size from millimeters to several centimeters. Gross Pathology - Intracortical tumors that thicken and expand the gyri. The glioneuronal component often has a viscous consistency together with single or multiple firmer nodules
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Clinical Issues • Most patients < 20 years Intractable, drug-resistant epilepsy Second most common tumor-associated temporal lobe epilepsy • Grows slowly, surgery usually curative • Recurrence, malignant transformation very rare
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Imaging findings Well-demarcated, triangular pseudocystic or bubbly cortical/subcortical mass in a young patient with longstanding complex partial epilepsy is highly suggestive of the diagnosis
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CT Findings NECT scans disclose a hypodense cortical / subcortical mass. Calcification - 20% of cases. Intratumoral hemorrhage - rare. Focal bony scalloping or calvarial remodeling is common with tumors adjacent to the inner table of the skull.
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MRI findings Wedge-shaped cortical/subcortical mass • "Points" toward ventricle • Multicystic / septated bubbly appearance ○ Hyperintense on T2WI ○ Rim of hyperintensity on FLAIR ○ Edema absent ○ Usually doesn't enhance
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14yrs/MC with long standing complex partial seizures
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Differential Diagnosis Focal cortical dysplasia Ganglioglioma Multinodular and vacuolating neuronal tumor of the cerebrum (MVNT) Angiocentric glioma Bubbly appearance of DNET and FLAIR hyperintense rims are helpful distinguishing features
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Rosette-Forming Glioneuronal Tumor Rare, slow growing glioneuronal tumor occurring only in the fourth ventricle Pineal region is the second most common site Clinical Issues RGNT is a tumor of young and middle-aged adults. Typical presenting symptoms are headache and ataxia.
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Pathologic hallmark of RGNT is its biphasic histology. Two distinct components are present. One consists of uniform neurocytes that form rosettes and/or perivascular pseudorosettes. Other is astrocytic and resembles pilocytic astrocytoma
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Imaging Heterogeneous-appearing mass centered within the fourth ventricle or vermis is the typical finding Multicystic appearance with hemorrhage on T2* (GRE or SWI), blood-fluid levels, and calcification is common. Moderate peripheral/heterogeneous pattern of enhancement CSF dissemination - imaging the complete neuraxis is appropriate whenever an RFGT is suspected.
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PAPILLARY GLIONEURONAL TUMOR Well-circumscribed, clinically indolent tumor of the cerebral hemispheres affecting young adults. WHO grade I neoplasm. Biphasic tumors - astrocytic and neuronal elements.
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PAPILLARY GLIONEURONAL TUMOR Distinguishing feature of PGNT Glial Fibrillary Acidic Protein (GFAP) positive neoplastic glial cells lining hyalinized vascular pseudopapillae. Synaptophysin positive interpapillary collections of neurocytes, large neurons, and intermediate sized ganglioid cells are present.
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Imaging findings – nonspecific Most tumors are located in the deep periventricular white matter Well-circumscribed cyst with enhancing nodule Mixed cystic and solid Completely solid masses Major differential diagnosis is ganglioglioma, which has a virtually identical imaging appearance to PGNT
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DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR Rare primary glioneuronal neoplasm that is characterized by widespread, plaque-like leptomeningeal growth. Tumors often exhibit an oligodendroglial-like cytology - Diffuse Oligodendroglial-like Leptomeningeal Neoplasm or Leptomeningeal Oligodendrogliomatosis. No WHO grade has been assigned to DLGNTs
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Clinical Issues Age at presentation varies from 2-7 years Signs and symptoms of slowly developing communicating hydrocephalus (i.e., headache, nausea, vomiting, and papilledema) are typical. CSF examination - elevated protein but no malignant cells.
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Imaging findings Moderate to severe obstructive hydrocephalus is common. Innumerable small cystic T2/FLAIR hyperintense nodules coating the pial surface of the spinal cord and brain especially in/around the cerebellar folia are typical T1 C+ sequences - Thickening and enhancement of the intracranial and spinal leptomeninges
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Differential diagnosis Pyogenic or tubercular meningitis may cause extensive leptomeningeal thickening and enhancement but rarely presents as multifocal leptomeningeal T2/FLAIR hyperintense cysts. Neurocysticercosis is rarely as diffusely disseminated as DLGNT. Cystic metastases from intracranial primary neoplasms (e.g., choroid plexus tumors) can mimic DLGNT.
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NEURONAL TUMORS Tumors that exhibit exclusive ganglion cell or neurocytic differentiation Gangliocytoma Neurocytoma Multinodular and vacuolating neuronal tumor of the cerebrum Dysplastic Cerebellar Gangliocytoma , A/K/A Lhermitte-Duclos disease (LDD)
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Gangliocytoma (GCyt) WHO grade I neoplasms Benign, well-circumscribed neoplasm that contains only differentiated ganglion cells. No glial component is present Etiology - Developmental tumor Location – 3/4 of supratentorial GCyts occur in the temporal lobe. Other sites - brainstem, sellar region, and spinal cord.
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Gross - GCyts can be solid or mixed solid and cystic lesions. Microscopically Bizarre-appearing mature ganglion cells. Mitoses are few or absent. CD34, a stem cell marker, is positive in the majority of GCyts..
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Clinical Issues Children and young adults under the age of 30 years. Present with pharmaco-resistant epilepsy. GCyts grow slowly. Surgical resection is generally curative and results in long-term progression-free survival
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Imaging findings NECT - Mixed density containing both cystic and solid components. Calcification is common (1/3) Hemorrhage and necrosis are absent. MRI - GCyts are hypo- to isointense relative to cortex on T1WI and hyperintense on T2/FLAIR Variable enhancement in the solid portions of the tumor
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29 / M with seizures
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Differential diagnosis Ganglioglioma - cortical, epilepsy-inducing tumors with both a cystic and enhancing solid component Cortical dysplasia - causes of refractory epilepsy in young patients, follows gray matter on all sequences and does not enhance
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MULTINODULAR AND VACUOLATING TUMOR OF THE CEREBRUM 2016 WHO considers MVNT a pattern of gangliocytoma True neoplasm vs. quasi-tumor (malformation, hamartoma) Benign, nonaggressive (WHO grade I)
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Pathology • Discrete, coalescent vacuolated nodules • Deep cortical ribbon/subcortical white matter • Small neurons and/or dysplastic ganglion cells Clinical Issues • Often found incidentally on imaging • Most common = Headache, Seizure
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Imaging Features • Multiple discrete small T2/FLAIR hyperintense nodules - Tiny "bubbles" • Along undersurface of cortex ○ Form cup around cortex ○ Ribbon of U-shaped hyperintensity • Normal MRS, DWI Differential Diagnosis • DNET (larger, surface of cortex)
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48/M with headache. H/O of cavernoma resection in contralateral hemisphere
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DYSPLASTIC CEREBELLAR GANGLIOCYTOMA Benign cerebellar mass composed of dysplastic ganglion cells. Dysplastic cerebellar gangliocytoma is also known as Lhermitte-Duclos disease (LDD). Granular cell hypertrophy Granulomolecular hypertrophy of the cerebellum Cerebellar hamartoma Diffuse ganglioneuroma Gangliomatosis of the cerebellum
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Etiology - Sporadic / Association of LDD with CS favors a hamartomatous origin Genetics - PTEN germline mutation that results in cellular proliferation of ectodermal, mesodermal, and endodermal tissues. CS is characterized by multiple hamartomas and malignant neoplasms.
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Location - always infratentorial, usually involving the cerebellar hemisphere or the vermis. Large lesions involve both. The brainstem is a rare site for LDD. Size and Number - Dysplastic cerebellar gangliocytomas often become very large, displacing the fourth ventricle and causing obstructive hydrocephalus
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The gross appearance of LDD is a tumor-like mass that expands and replaces the normal cerebellar architecture . On cut section, the cerebellar folia are markedly widened and have a grossly "gyriform" appearance
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Adults - 20-40 years. No sex predilection Presentation - Patients may be asymptomatic or present with symptoms of increased intracranial pressure Treatment Options Shunting or surgical debulking - symptomatic patients with hydrocephalus. LDD blends gradually into normal cerebellar tissues - complete resection is difficult and the complication rate is high.
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Imaging findings CT Findings Hypodense on NECT. Mass effect with compression of the fourth ventricle, effacement of the cerebellopontine angle cisterns, and obstructive hydrocephalus is common. Calcification is rare. Necrosis and hemorrhage are absent. CECT - no appreciable enhancement
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MR Findings - expansile cerebellar mass T1WI - Linear hypointense bands T2WI - Tiger stripe pattern of alternating inner hyperintense and outer hypointense layers in enlarged cerebellar folia GRE, SWI - demonstrates prominent venous channels surrounding the grossly thickened folia. T1 C+ - linear enhancement of these abnormal veins in between the folia. DWI - restricted diffusion- reflecting the hypercellularity and increased axonal density characteristic of LDD.
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PWI - increased relative CBV , - prominent enlarged interfolial veins. MRS - normal or slightly reduced NAA and normal Cho:Cr ratios. A lactate doublet may be present. F-18 FDG PET imaging - hypermetabolic . High-definition fiber tractography - powerful surgical planning tool in LDD, allowing maximal lesion resection without damage
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DifferentiaL Diagnosis Imaging findings of LDD are so characteristic that the diagnosis can usually be established without biopsy confirmation. While the suggested differential diagnosis has included medulloblastoma and subacute cerebellar infarction Nonenhancing unilateral cerebellar mass in a middle-aged patient that demonstrates a prominent tiger stripe pattern on MR is typical of LDD
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CENTRAL NEUROCYTOMA Benign-acting intraventricular neoplasm of young adults, originally thought to be an oligodendroglioma subtype, is now recognized as a tumor of neuronal lineage and has been given the name central neurocytoma CNC is a well-differentiated neuroepithelial tumor with mature neurocytic elements WHO grade II neoplasm
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Etiology The precise origin of CNCs is unknown. Bipotential precursor cells of the periventricular germinal matrix are capable of both neuronal and glial differentiation Size and Number CNCs vary in size from small to huge and extending through the foramen of Monro to involve the contralateral ventricle.
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Location CNCs are tumors of the lateral ventricle body, usually attached to the septi pellucidi and arising near the foramen of Monro
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Clinical issues Most common primary intraventricular neoplasm of young and middle-aged adults. Symptoms are usually those of increased intracranial pressure. Headache, mental status changes, and visual disturbances are common. Focal neurologic deficits are rare
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Imaging Findings CT Findings Mixed-density solid and cystic intraventricular neoplasm that is attached to the septi pellucidi. Obstructive hydrocephalus is common. Calcification + Frank intratumoral hemorrhage occurs but is rare CECT - moderately strong but heterogeneous enhancement on
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MR Findings. CNCs are heterogeneous masses that are mostly isodense with gray matter on T1WI. Intratumoral cysts and prominent vascular "flow voids" are common. Bubbly Appearance on T2WI is typical .CNCs are heterogeneously hyperintense on FLAIR and demonstrate moderate to strong but heterogeneous enhancement following contrast administration Bubbly mass in the body or frontal horn of the lateral ventricle is classic for CNC
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18yrs/F with progressively inccreasing headache in the past one month
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Differential diagnosis Subependymoma Subependymal giant cell astrocytoma Intraventricular metastasis Intraventricular oligodendroglioma
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Extraventricular Neurocytoma Neoplasms that resemble central neurocytomas reported outside the ventricular system are termed as extraventricular neurocytoma. WHO grade III Young adults. MC presenting symptom – Epilepsy Location - Cerebral hemispheres or parasellar region
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Imaging findings EVNCTs vary widely in imaging appearance. T2 hyperintense bubbly appearance - resemble CNCs or DNETs Cyst + nodule configuration similar to ganglioglioma. Large, heterogeneous, enhancing parenchymal neoplasms that are difficult to distinguish from high-grade astrocytoma, primitive neuroectodermal tumor (PNET), or supratentorial ependymoma and probably represent "atypical" EVNCTs
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Thank you !